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Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common inflammatory neuropathy that can be progressive, stepwise progressive, or relapsing and remitting., Objectives: To further evaluate the long-term safety and tolerability of immune globulin intravenous, 10% caprylate-chromatography purified immune globulin intravenous in CIDP., Design: Randomized multicenter trial., Setting: Hospitals and outpatient clinics., Patients: Adults with CIDP (n = 117) [corrected]., Interventions: Immune globulin intravenous, 10% caprylate-chromatography purified (2 g/kg of body weight) or placebo was infused as a baseline loading dose, followed by a maintenance dose (1 g/kg) every 3 weeks for up to 24 weeks. PATIENTS who responded were rerandomized into a double-blind extension phase of immune globulin intravenous, 10% caprylate-chromatography purified (1 g/kg) or placebo every 3 weeks for up to 24 weeks. PATIENTS who relapsed during the extension phase were withdrawn from the study., Main Outcome Measures: Additional analyses of safety and tolerability., Results: Overall, 113 patients and 95 patients were exposed to immune globulin intravenous, 10% caprylate-chromatography purified and placebo, respectively. Exposure to immune globulin intravenous, 10% caprylate-chromatography purified was approximately twice that of placebo (1096 vs 575 infusions). Most maintenance dose courses were administered over 1 day in the immune globulin intravenous, 10% caprylate-chromatography purified (89.1% of 783 dose courses) and placebo (91.1% of 359 dose courses) groups. The most common drug-related adverse events (AEs) with immune globulin intravenous, 10% caprylate-chromatography purified were headache (4.0 per 100 infusions) and pyrexia (2.4 per 100 infusions). Five drug-related serious AEs (pulmonary embolism, pyrexia, vomiting, and 2 headache events) were reported in 3 patients (2.7%) exposed to immune globulin intravenous, 10% caprylate-chromatography purified. The incidence of drug-related serious AEs was higher after loading dose infusions than after maintenance dose infusions (4 AEs vs 1 AE). Age, weight, CIDP severity, and previous immune globulin intravenous exposure had no substantial effect on the percentage of patients with AEs, including serious AEs., Conclusion: Data support a favorable safety and tolerability profile for administration of immune globulin intravenous, 10% caprylate-chromatography purified as CIDP maintenance therapy., Trial Registration: clinicaltrials.gov Identifier: NCT00220740.

Background: Celiac disease (CD) is increasingly recognized in North America and is associated with a peripheral neuropathy., Objective: To report the clinical characteristics and skin biopsy results in patients with CD and small-fiber neuropathy symptoms., Design: Case series., Setting: Academic peripheral neuropathy clinic., Patients: Eight patients with CD and neuropathy symptoms. Intervention Three-millimeter punch biopsy using the panaxonal marker protein gene product 9.5 to assess epidermal nerve fiber (ENF) density and a gluten-free diet., Main Outcome Measure: Clinical data and ENF density., Results: All patients had asymmetric numbness and paresthesias. Three had more prominent involvement of hands than feet, and 3 had facial numbness. Celiac disease was diagnosed in 5 after their neuropathy began. The following serum antibody levels were elevated: tissue transglutaminase (n = 6), IgA gliadin (n = 4), and IgG gliadin (n = 7). Results of nerve conduction studies were normal in 7 patients. One patient had mildly reduced sural amplitudes. The ENF density was reduced in 5 patients. The ENF density was at the low limit of the normal range in 3 additional patients, 2 of whom had morphologic changes in axons. Three patients had decreased ENF density at the thigh or forearm, which was more severe than at the distal leg, compatible with a non-length-dependent process. Four reported improvement with a gluten-free diet. One had no improvement after 4 months. Symptoms developed in 2 while receiving a gluten-free diet., Conclusions: Patients with CD may have a neuropathy involving small fibers, demonstrated by results of skin biopsy. The pattern of symptoms, with frequent facial involvement and a non-length-dependent pattern on skin biopsy findings, suggests a sensory ganglionopathy or an immune-mediated neuropathy. Improvement of symptoms in some patients after initiating a gluten-free diet warrants further study.

Background: Current electrodiagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) are research oriented favoring specificity over sensitivity. Application of such criteria in clinical practice may miss the diagnosis in potentially treatable patients., Objectives: To analyze the electrophysiologic abnormalities in a cohort of patients with clinically defined CIDP, to compare these data with published electrodiagnostic criteria, and to identify a set of abnormalities that is shared by all patients with CIDP., Design: Retrospective medical record review., Setting: Academically based neuromuscular clinic. Patients Fifteen patients with clinically diagnosed relapsing sensorimotor CIDP., Interventions: Administration of intravenous immunoglobulin or prednisone., Main Outcome Measures: Electrodiagnostic studies., Results: All patients had electrodiagnostic abnormalities in at least 3 nerves with possible partial conduction block or demyelinating range abnormalities in at least 1 nerve. The diagnostic sensitivities of 5 published CIDP criteria were as follows: the Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force (40%), Saperstein et al (47%), Nicolas et al (53%), Hughes et al for the Inflammatory Neuropathy Cause and Treatment Group (60%), and Thaisetthawatkul et al (70%)., Conclusions: Current electrodiagnostic criteria for CIDP are insensitive and may fail to diagnose the condition in a substantial number of patients. More inclusive criteria that allow identification of patients in routine clinical practice are needed.

Background: Multifocal acquired demyelinating sensory and motor neuropathies are considered autoimmune and responsive to immunotherapy. In the absence of demyelination, however, they are considered idiopathic if no other cause is found., Objective: To determine whether patients with multifocal acquired sensory and motor neuropathy of an otherwise unknown cause have antiganglioside antibodies, regardless of whether they are classified as demyelinating or axonal, indicating a possible immune-mediated origin., Patients and Methods: Serum samples from 25 patients with multifocal acquired sensory and motor neuropathy of an otherwise unknown cause were tested for antibodies to gangliosides using an agglutination immunoassay. Reactive serum samples were further tested by enzyme-linked immunosorbent assay against individual gangliosides. Electrophysiologic studies were reviewed for evidence of demyelination., Results: Increased levels of ganglioside antibodies were detected in 12 (48%) of the 25 patients using the agglutination immunoassay, and in 7 (58%) of the 12 agglutination-positive patients by the enzyme-linked immunosorbent assay. Serum samples from these 7 patients had IgG antibodies to 1 or more gangliosides; none had elevated levels of IgM antiganglioside antibodies. Three of the patients fulfilled 2 of the American Academy of Neurology electrophysiologic criteria for demyelination, but none fulfilled the 3 of the 4 possible criteria required for the diagnosis of demyelinating neuropathy. A sural nerve biopsy specimen in 2 patients revealed axonal degeneration., Conclusion: Multifocal sensory and motor neuropathies of an otherwise unknown cause may be associated with antiganglioside antibodies, regardless of whether they exhibit demyelinating features.